Physical activity levels of patients prior to acute coronary syndrome – Experience at a tertiary care hospital in Sri Lanka

Objectives Physical inactivity is associated with high cardiovascular risk. The purpose of this study was to study physical activity levels of patients with acute coronary syndrome (ACS). Methodology A total of 504 patients, from the Professorial Unit of the Colombo South Teaching Hospital completed the International Physical Activity Questionnaire (IPAQ). IPAQ is a validated questionnaire used internationally to objectively measure physical activity. Both the total volume and the number of sessions of activity are included in the IPAQ. Populations are divided into three levels based on physical activity levels: low, moderate and high activity. Results Out of 504 patients, 128 (25.1%) were highly active, 87 (17.1%) were minimally active and 289 (56.7%) were found be inactive. When considering mets per week 134 (26.3%) spent less than 1000 mets/week. Physical activity levels of men and women were similar (p = 0.06). There was a no significant association between body mass index (BMI) with total IPAQ score (p = 0.11). There was no difference in the physical activity levels of patients presenting with different types of ACS: ST-Elevation Myocardial Infarction (STEMI), non-ST-elevation myocardial infarction (NSTEMI) or unstable angina (UA) (p = 0.36). The activity levels did not affect the severity of ACS assessed by Thrombolysis In Myocardial Infarction (TIMI) scores. (NSTEMI/UA p = 0.24, STEMI p = 0.10). Conclusion In Sri Lanka, a majority of patients with ACS were physically inactive. Physical inactivity is one of the remediable major risk factors of ACS and an active life style should be promoted.

An intervention study to monitor weight gain in infants using a home based complementary food recipe and a hand blender.

OBJECTIVES: To assess the efficacy of a home made energy dense weaning food, containing 110-130 kcal (26-30kJ) per 100 ml on prevention of growth faltering during infancy. METHODOLOGY: Infants attending four child health welfare clinics in the Medical Officer of Health (Ragama) area were recruited at the age of 4 months. The intervention group received a specially designed hand blender, recipe and advice to prepare a weaning food. The control group received weaning foods without any intervention. They were followed up monthly up to the age of 12 months. RESULTS: 152 infants completed the study (83 from intervention group). The infants in the intervention group gained significantly more weight than the control group (intervention group 2.43 +/- 0.72 kg, control group 2.02 +/-0.62 kg, p = 0.0002). Both groups showed a drop in the Z score for mean weight for age during the study period but this was less marked in the intervention group. CONCLUSIONS: A high energy density home made complementary food was effective in improving the weight gain of infants during the weaning period.

Experience with the oral iron chelator deferiprone in transfusion-dependent children.

OBJECTIVE: To establish efficacy and safety of deferiprone. DESIGN: Prospective study. SETTING: The Lady Ridgeway Hospital for Children, Colombo. PATIENTS: Transfusion-dependent children in the age group 1 to 15 years. INTERVENTION: Patients were given 75 mg/kg/day of deferiprone orally in divided doses. MEASUREMENTS: Efficacy of deferiprone therapy was assessed by 4 to 6 monthly serum ferritin (SF) assays. Safety of therapy was assessed by 4-weekly white cell counts and serum alanine aminotransferase (ALT) levels. The Z-score was used to assess the significance of the difference between the mean initial and final SF level. RESULTS: 82 patients received deferiprone therapy for a mean duration of 30 +/- 14 months. Initial SF levels ranged from 1115 to 12,165 micrograms/l with a mean of 5156 +/- 2631 micrograms/l. Final SF levels ranged from 312 to 15,285 micrograms/l with a mean of 2809 +/- 2380 micrograms/l (Z score 5.99; p < 0.001). Two (2.4%) children developed agranulocytosis which reverted to normal on discontinuation of treatment. 41 (50%) developed arthropathy and in 17 this was severe enough to require discontinuation of therapy. Serum ALT levels were raised in 35 (43%) patients but reverted to pretreatment values or lower despite continuation of deferiprone therapy. There was one death in a 9-year old child who developed diabetes mellitus and heart failure despite deferiprone therapy for 3 years. CONCLUSIONS: A final SF level < 2500 micrograms/l was achieved in 52% children. Severe arthropathy and agranulocytosis may necessitate permanent discontinuation of therapy.

A preliminary study of the impact of long-term psychotic disorder on patients' families.

OBJECTIVE: To assess impact of long term psychotic disorders on caregivers. DESIGN: A questionnaire based, interviewer administered, cross-sectional survey using the translated version of a Burden Assessment scale (BAS). SETTING: Outpatient clinic of the University Psychiatry Unit, National Hospital, Sri Lanka. SAMPLE: 50 caregivers of patients suffering from psychotic disorders for more than 2 years. MEASUREMENTS: The BAS was administered to 50 caregivers to assess degree of distress and domains of concerns. RESULTS: 60% of caregivers felt very anxious and depressed. 54% experienced a financial decline, and 82% felt responsible for meeting the entire financial needs of the patient. 54% felt that their workload increased due to the illness. 58% of the caregivers were parents. CONCLUSIONS: Caregivers of patients with long term psychotic disorders are distressed, and have several concerns. Interventions focused on these will relieve the distress of caregivers and help patients. A majority of caregivers are parents. This has future implications, as many patients are not capable of independent living.

Meckel Gruber syndrome--a single gene cause of recurrent neural tube defects.

Meckel Gruber syndrome (MGS), an autosomal recessive disorder characterised by posterior encephalocoele, multicystic kidneys and post-axial polydactyly should be recognised by obstetricians and paediatricians to counsel parents regarding the 25% recurrence risk. We report a consanguineous family with MGS affecting three infants.

Geo-helminth infections in a rural area of Sri Lanka.

School children carry the heaviest burden of morbidity due to intestinal helminth infection. The objective of this investigation was to study geo-helminth infections in 349 school children aged 6 to 13 years living in a rural area of Sri Lanka. Stool samples were examined by direct saline smear in an initial survey to determine the prevalence of intestinal parasitic infections and thereafter the children were followed up over a two year period with cross sectional surveys of stool samples being carried out at yearly intervals. Following collection of a stool sample, all the subjects were treated with mebendazole 500 mg as a single dose. Weights and heights were measured using standardized procedures. 2 ml of venous blood were collected from each subject under aseptic conditions to determine hematological indices. The prevalence of geo-helminth infections was low, and the prevalence declined during the two-year period from 5.4% in 1997 to 2.2% in 1998 and 2.0% in 1999 following yearly mass anti-helminth treatment. The incidence density was 0.021 cases per child year. The reduction in the prevalence from the baseline to the second survey is probably due to the reduction of the reservoir of infection among children as a result of mass treatment at baseline. The prevalence of infection during the second and third surveys were almost the same probably due to infections originating from other segments of the untreated population.

A trial of deferiprone in transfusion-dependent iron overloaded children.

OBJECTIVE: To determine the efficacy and safety of deferiprone. DESIGN: Prospective study. SETTING: 5 paediatric medical units at the Lady Ridgeway Hospital for Children (LRHC), Colombo. PATIENTS: Transfusion-dependent iron overloaded children in the age group 2 to 15 years. INTERVENTION: Patients were given a total daily dose of 75 mg/kg of deferiprone orally in divided doses. MEASUREMENTS: Efficacy of deferiprone therapy was assessed by 4-monthly serum ferritin assays using the ELISA technique. Safety of deferiprone therapy was assessed by 4-weekly white cell counts, platelet counts and serum transaminase levels. The Z-test was used to assess the significance of the difference between the mean initial serum ferritin level and the mean subsequent serum ferritin level. RESULTS: 54 patients received deferiprone therapy for a mean duration of 9 +/- 3 months. Initial serum ferritin levels ranged from 1500 to 10,700 ng/ml with a mean of 5743. Subsequent serum ferritin levels, obtained in 48 patients ranged from 740 to 7300 ng/ml with a mean of 3558 (p < 0.001). In 47 of the 48 patients subsequent serum ferritin levels were lower than initial levels. One child developed severe neutropaenia, which reverted to normal on discontinuation of treatment. 11 children developed arthropathy, which responded to ibuprofen therapy combined in some cases with a reduction of the dose of deferiprone to 50 mg/kg/day. Serum transaminase levels were raised in 5 patients but reverted to pretreatment values or lower despite continuation of deferiprone therapy. CONCLUSIONS: Deferiprone is a safe and effective oral iron-chelating agent which can be used, under strict supervision, in transfusion-dependent iron overloaded children.

Familial adenomatous polyposis.

Familial adenomatous polyposis (FAP) is a rare disorder associated with less than 1% of colorectal carcinomas (CRCs). Since FAP is a potentially preventable cause of CRC clinicians should have an adequate knowledge of it to identify the disease and to manage the patient and family. FAP is an autosomal dominant inherited disorder characterised by the development of more than a hundred adenomatous polyps in the colon and rectum which can undergo malignant change. Children of an affected individual are at 50% risk of inheriting the predisposing gene. After the identification of an index patient, genetic testing in combination with the detection of extra-colonic manifestations allows more accurate identification of family members likely to have the faulty gene, enabling the targetting of screening and preventive surgery only to those at risk. FAP also provides insights into the development, progression and prevention of sporadic CRC.

Attitudes towards genetic counselling and testing among medical students and newly qualified doctors.

OBJECTIVES: To determine knowledge about four genetic disorders (Down's syndrome (DS), haemophilia (haem), spinal muscular atrophy type 1 (SMA1) and Huntington's disease (HD)), attitudes towards counselling, acceptability of prenatal diagnosis and termination of pregnancies affected with these conditions. DESIGN: Questionnaire survey of a cohort of medical students and newly qualified doctors. SETTING: Faculty of Medicine, University of Ruhuna. RESULTS: 227 completed questionnaires (111 fourth year and 86 final year students, and 30 demonstrators) were analysed. Awareness of DS and haem, was higher than of SMA1 and HD, and was highest among the demonstrators. Over 80% of the cohort would not counsel directively about future pregnancies and would discuss the diseases with the family or at risk individuals. Prenatal diagnosis was found acceptable for DS, haem and SMA1 by a majority of the cohort. Attitudes to termination of affected pregnancies varied, 88%, 77%, 55% and 36% finding it acceptable for DS, SMA1, haem, and HD respectively, provided legal terminations were available and termination was requested by parents. CONCLUSIONS: This cohort of students and doctors appear to accept the principles of clinical genetics involving non-directive counselling, prenatal diagnosis and in some disorders, termination of pregnancy.

Infantile Cronkhite-Canada syndrome.

Since 1955, numerous cases of Cronkhite-Canada syndrome have been reported in adults. We present here the first Asian report of the infantile Cronkhite-Canada syndrome. While the prognosis in adults is variable, in children, it is less optimistic.